Isolated Trigeminal Neuralgia Presented Due to Cerebellopontine Angle Epidermoid Cyst

Trigeminal neuralgia (TN) is a paroxysmal shock like pain restricted to the innervations of the areas of one or more trigeminal branches. The pathogenesis of TN is uncertain and typically is idiopathic, but it may be due to a structural lesion. Various etiologies such as vascular anomaly, tumor, infectious agents, and multiple sclerosis have been implicated as possible causes. Here we report two young patients diagnosed with trigeminal neuralgia secondary to epidermoid cyst at the cerebellopontine angle.

A case report of recurrent Meckel Gruber syndrome / 대한산부인과학회지

Meckel Gruber syndrome consisting of an occipital encephalocele, polycystic kidney disease and polydactyly is a rare autosomal recessive disorder with a recurrence risk of 25%. Targeted ultrasonography in late embryonic or early fetal stages of pregnancy has great importance in diagnosis and management of affected pregnancy in high risk groups due to incomplete genetic mapping of meckel syndrome gene (MKS). We present a case of prenatal diagnosis at 14 weeks' gestational age of Meckel Gruber syndrome in a woman, who experienced same disorder in her previous pregnancy.

Clinical and Pathological Characteristics of Four Korean Patients with Limb-Girdle Muscular Dystrophy type 2B

Limb-girdle muscular dystrophy type 2B (LGMD2B), a subtype of autosomal recessive limb-girdle muscular dystrophy (ARLGMD), is characterized by a relatively late onset and slow progressive course. LGMD2B is known to be caused by the loss of the dysferlin protein at sarcolemma in muscle fibers. In this study, the clinical and pathological characteristics of Korean LGMD2B patients were investigated. Seventeen patients with ARLGMD underwent muscle biopsy and the histochemical examination was performed. For the immunocytochemistry, a set of antibodies against dystrophin, alpha, beta, gamma, delta-sarcoglycans, dysferlin, caveolin-3, and beta-dystroglycan was used. Four patients (24%) showed selective loss of immunoreactivity against dysferlin at the sarcolemma on the muscle specimens. Therefore, they were classified into the LGMD2B category. The age at the onset of disease ranged from 9 yr to 33 yr, and none of the patients was wheelchair bound at the neurological examination. The serum creatine kinase (CK) was high in all the patients (4010-5310 IU/L). The pathologic examination showed mild to moderate dystrophic features. These are the first Korean LGMD2B cases with a dysferlin deficiency confirmed by immunocytochemistry. The clinical, pathological, and immunocytochemical findings of the patients with LGMD2B in this study were in accordance with those of other previous reports.

Evaluation of Cervical Spondylosis by Cervical MRI in Patients with Amyotrophic Lateral Sclerosis

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease involving upper and lower motor neurons, which leads to respiratory paralysis and death. Although ALS has been well known to be combined with cervical spondylosis, its frequency has not been established. The purpose of this study is to investigate the frequency of the cervical spondylosis in patients with ALS. METHODS: We evaluated the frequency and degree of spinal stenosis in ALS patients using cervical MRI. The study groups were composed of 30 ALS patients and 30 healthy controls. Using the T2-weighted cervical MRI, we measured the anteroposterior diameters of the cord and canal in the sagittal and axial views at the narrowest and C3-4 levels, and calculated the ratios of cord/canal (CCr). We also measured the areas of the cord and canal in the axial views at the narrowest and C3-4 levels. RESULTS: Of the 30 patients, 20 (66.7%) had cervical spondylosis. On sagittal and axial view, the average CCr at the narrowest / C3-4 level were 71.2 +/- 10.4 / 67.8 +/- 9.6% and 73.1 +/- 9.3 / 71.3 +/- 8.2% in ALS, and 59.2 +/- 4.4 / 56.7 +/- 5.9% and 62.5 +/- 5.6 / 60.4 +/- 6.4% in controls. The area of spinal cord at the narrowest / C3-4 level was 69.1 +/- 9.8 / 70.3 +/- 8.7 mm2 in ALS, and 78.4 +/- 11.3 / 78.9 +/- 10.8 mm2 in controls. The area of canal at the narrowest / C3-4 level was 149.7 +/- 12.5 / 151.5 +/- 7.8 mm2 in ALS, and 172.5 +/- 12.5 / 173.8 +/- 11.6 mm2 in controls. CONCLUSIONS: Our results showed the high frequency of cervical spondylosis and spinal cord atrophy in ALS.

Clinical Analysis of the Laryngeal Leukoplakia / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Leukoplakia is a descriptive term designating a white patch or plaque of the mucosa and must be complemented by histology. The aim of this study was to analyze the laryngeal leukoplakia clinically. MATERIALS AND METHOD: 48 patients were included from 1995 to 2001. All presented themselves with laryngeal leukoplakia in telescopic examination. The available medical record are age, sex, duration of smoking, histopathological finding, treatment and follow up result. RESULTS: The patients were composed of 46 males and 2 females. There were 7 patients (15%) with specific diagnostic lesion (polyps, lymphoma, tuberculosis, chronic inflammation and papilloma), 19 patients (40%) with simple hyperplasia or hyperkeratosis, 4 patients (8%) with mild dysplasia, 2 patients (4%) with moderate dysplasia, 2 patients (4%) with severe dysplasia, 2 patients (4%) with carcinoma in situ, and 1 patient (2%) with microinvasive carcinoma and 11 patients with invasive squamous cell carcinoma. Rebiopsy was performed to 10 cases during follow up period. CONCLUSION: The results of our study show that the leukoplakia was presented in 15% of all cases with specific diagnostic lesion and that the patient was treated according to their specific diagnosis. Almost all of the case with precancerous conditions were cured with cord stripping, cordectomy or radiation therapy.

A Case of Recurred Malignant Lymphoma Originating Non-Head and Neck Area during Chemotherapy / 대한이비인후과학회지

Malignant lymphoma is an uncommon non-squamous cell tumor of the larynx. Clinical suspicion and deep structural pathologic biopsy is very important in diagnosis. Chemotherapy or combined chemoradiotherpy is the routine treatment method. We experienced a case of recurred malignant lymphoma originating from a non-head and neck area during chemotherapy in a 35-year-old man who complained of dysphagia and hoarseness. This laryngeal lymphoma was excised by CO2 laser and chemotherpy.

Effect of Photostimulation Using Low-Level Laser Therapy on Wound Healing in Induced Mouse Skin Wound / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: This study was conducted to investigate the healing effect of the low-level laser irradiation on wound healing in vivo using DPSS laser (532 nm) and Diode laser (660 nm). MATERIALS AND METHOD: Each mouse received dorsal, full-thickness round incision (=2 cm) and daily laser irradiation (4 J/cm2) was done before sacrifice. On sacrifice at 3, 7, 10 days, the wound was excised, then wound closure and histologic stages were measured, and standardized. RESULTS: The percentages of wound closure in DPSS laser, Diode laser, control were 33.2+/-2.4, 34.2+/-3.5, 24.0+/-2.7 at day 3, 64.8+/-3.5, 72.2+/-2.8, 42.8+/-5.0 at day 7 and 82.2+/-7.9, 87.2+/-3.7, 71.4+/-4.0 at day 10, respectively, with p<0.05. Histological evaluation showed that laser irradiation enhanced wound epithelialization, cellular content deposition, granulation tissue formation, collagen deposition and neovascularization in the laser-treated wounds as compared to the control group. CONCLUSION: Low-level laser irradiation at 532 nm and 660 nm significantly enhanced cutaneous wound healing effect in the wounded mouse model. Further investigation of the mechanism of low-level laser therapy in primary wound healing is warranted.

Urodynamic Study in Patients with Myelitis

BACKGROUND: Voiding disturbance is one of the frequent symptoms of myelitis. Clinical manifestations of urinary disturbance in the patients with myelitis are results of the specific neurological lesion of the spinal cord, but can be varied. To determine the relationship between the neurological spinal cord level and bladder dysfunction, we nalyzed the urodynamic study in 30 patients with myelitis with urinary symptoms. Neurological examination and spinal MR imaging determined the clinical and radiological spinal cord levels. Urodynamic findings were classified as detrusor hyperreflexia (DH), detrusor-external sphincter dyssynergia (DSD), detrusor areflexia/hyporeflexia (DA) or normal. We analyzed the correlation between the neurological spinal level and urodynamic findings. RESULTS: Among the 30 patients, 14 patients were revealed DA, 8 were DH, 2 were DSD, and 6 were normal. Of 22 cervicothoracic lesions, 9 had DA, 7 had DH and 2 had DSD, but 4 had normal urodynamic findings. Four lumbar and sacral lesions had DA. CONCLUSIONS: The Urodynamic study finding in the lumbosacral lesion revealed DA, but, in those with cervicothoracic lesion are varied including DA, DH, DSD and normal. Urodynamic evaluation may provide more information for management of neurourological dysfunction.

Urodynamic Study in Patients with Myelitis

BACKGROUND: Voiding disturbance is one of the frequent symptoms of myelitis. Clinical manifestations of urinary disturbance in the patients with myelitis are results of the specific neurological lesion of the spinal cord, but can be varied. To determine the relationship between the neurological spinal cord level and bladder dysfunction, we nalyzed the urodynamic study in 30 patients with myelitis with urinary symptoms. Neurological examination and spinal MR imaging determined the clinical and radiological spinal cord levels. Urodynamic findings were classified as detrusor hyperreflexia (DH), detrusor-external sphincter dyssynergia (DSD), detrusor areflexia/hyporeflexia (DA) or normal. We analyzed the correlation between the neurological spinal level and urodynamic findings. RESULTS: Among the 30 patients, 14 patients were revealed DA, 8 were DH, 2 were DSD, and 6 were normal. Of 22 cervicothoracic lesions, 9 had DA, 7 had DH and 2 had DSD, but 4 had normal urodynamic findings. Four lumbar and sacral lesions had DA. CONCLUSIONS: The Urodynamic study finding in the lumbosacral lesion revealed DA, but, in those with cervicothoracic lesion are varied including DA, DH, DSD and normal. Urodynamic evaluation may provide more information for management of neurourological dysfunction.

Metabolism of Valproate in Traumatic Brain Injury Patients / 대한간질학회지

PURPOSE: Traumatic brain injuries induce plasma clearance of several compounds. Phenytoin is usually used for the prevention of posttraumatic seizure, and it is known that its clearance is increased in traumatic brain injury patients. Valproate is another important antiepileptic drug for the prevention of posttraumatic seizure. We evaluated the metabolism of valproate in acute traumatic brain injury patients. METHODS: Fourteen traumatic brain injury patients were selected. They were given a loading dosage (15-20 mg/kg), and maintained with valproate. Trough serum valproate level was obtained during 7-20 days after an acute injury. RESULTS: The total clearance level of valproate was higher in acute traumatic brain injury patients than patients with chronic valproate use (7.70+/-1.36 ml/kg/hr vs. 9.05+/-1.91 ml/kg/hr, p<0.05). CONCLUSIONS: Acute traumatic brain injury results in the induction of valproate metabolism during 7-20 acute days, and it is related to the enhancement of multiple metabolic pathways.

A Case of Spinocerebellar Ataxia Type 7 with Torticollis

The spinocerebellar ataxia type 7 is an autosomal dominant neurodegenerative disorder with expansion of unstable CAG trinucleotide repeats in a gene on chromosome 3p, and is classified as autosomal dominant cerebellar ataxia type II. Extrapyramidal findings are uncommonly recognized in autosomal dominant cerebellar ataxia type II. A 27-year-old woman showed progressive ataxia, visual disturbance and torticollis. We report a case of genetically confirmed spinocerebellar ataxia type 7 with extrapyramidal finding.

A Familial Case of Myoclonus-Dystonia

BACKGROUND: Myoclonus-dystonia is a rare familial disease characterized by autosomal dominant inheritance, nonor slowly progressive axial myoclonus combined with dystonic posture, normal electroencephalography (EEG) finding, and dramatic response to alcohol intake. METHODS: Clinical manifestations were studied in a family with myoclonus-dystonia. Response to alcohol intake was investigated in affected adult patients. Brain magnetic resonance imaging (MRI) and other laboratory examination were performed in 3 patients. RESULTS: Eight (male: 5, female: 3) of the 14 biological family members through 4 generations were found to be affected by myoclonus-dystonia on neurological examinations. Another 5 members (male: 3, female: 2) were suspected to be affected in family history. All eight affected members showed axial myoclonus affecting the neck, trunk, and proximal muscles of the limbs. Six of them also had dystonia affecting the neck or the distal part of the arm. Myoclonus and dystonia were ameliorated dramatically after small dose of alcohol intake. Brain MRI, EEG study, and ophthalmologic examination showed no abnormalities. CONCLUSIONS: Our patients showed clinical features compatible with myoclonus-dystonia. This is the first Korean family with myoclonus-dystonia.

Clinical Analysis of Bilateral Vestibulopathy

From retrospective review of medical charts of patients diagnosed as bilateral vestibulopathy during the past seven years, this study investigated incidence, etiologies, symptoms, vestibular function test findings; electronystagmography (ENG) studies, rotatory chair testing, and posturography, and post-vestibular rehabilitation (VRT) follow-up results. we have an incidence of 1.2% of all the 3423 patients who have undergone vestibular function test. Ototoxicity was the first known etiology and we had a lot of patients of idiopathic bilateral vestibulopathy. Most patients in our study had experiences of sudden onset vertigo rather than slowly progressive symptoms. The most frequently complained symptoms were dysequilibrium and oscillopsia. Post-VRT courses were better in patients whose initial gain of vestibulo-ocular reflex(VOR) was high or ascending type.

The Effect of Initial Serum Neuron-Specific Enolase Level on Clinical Outcome in Acute Carotid Artery Territory Infarction

The prediction of functional outcome in patients with acute cerebral infarction depends on many factors. Various techniques have been applied to predict severity and outcome after cerebral infarction. Neuron-specific enolase (NSE) is a component of a specific brain enzyme and a useful marker of brain injury. We evaluated the relation between initial serum NSE level and short- and long-term clinical outcome in 59 patients with acute cerebral infarction and in 38 age-matched healthy controls. Serum NSE levels were determined in patients with carotid artery (CA) territory cerebral infarction within 24 hours of onset. Brain MRI was performed four to seven days after stroke. Patients were divided into two groups: large CA territory infarction with a lesion extending cortex (cortex group), and small subcortical CA territory infarction (subcortical group) with a lesion confined to the subcortical white matter. We compared the initial serum NSE levels of the two groups. National Institute of Health Stroke Scale (NIHSS) was determined at admission and seven days after onset and the modified Rankin's scale was used at the 3 months follow-up after onset. Serum NSE levels were significantly elevated in patients with acute cerebral infarction compared with the normal controls (13.88 +/- 5.47 ng/dl vs. 8.15 +/- 1.53 ng/dl, p < 0.05). The initial ( < 24 h) serum NSE level was higher in the cortical group than in the subcortical group (16.68 +/- 5.70 ng/dl vs. 10.98 +/- 3.34 ng/dl, p < 0.05). NIHSS on admission and on the 7th day correlated with the initial serum NSE level (p < 0.05), as were more severe functional outcomes, as determined 3 months after onset (p < 0.05). This study shows that initial serum NSE level may be a useful marker for severity in acute ischemic stroke, and that it may be well correlated with short-term and long-term functional outcomes.

The Effect of Subthalamic Nucleus Stimulation in Patients with Advanced Idiopathic Parkinson's Disease

BACKGROUND: To determine the efficacy and safety of subthalamic nucleus (STN) stimulation in patients with advanced Parkinson's disease (PD). METHODS: In 5 patients with PD, we evaluated the effect of bilateral STN stimula-tion. Using the Unified PD Rating Scale (UPDRS), Clinical Dyskinesia Rating Scale, Activities of Daily Living(ADL) Score and patient's diary, we evaluated the patients before and at one, three and 12 months after surgery. We examined the patients while they were drug "off" and "on". RESULTS: While patients were "off", stimulation induced a signifi-cant reduction in the UPDRS part III score by 46% at 12 months after the operation, compared to the baseline state. During drug "on" state, levodopa-induced dyskinesias were reduced by 88% at 12 months after the operation. Off-peri-od dystonia was reduced by 45% at 12 months after the operation. ADL scores also improved after the stimulation. Patients' diaries showed significant reduction in the "off" period while awake (73% reduction at 12 months). The daily dose of levodopa was reduced by 56% at 12 months after the operation. There was no significant complication related to the surgical procedure or electrical stimulation. CONCLUSIONS: We conclude that STN stimulation is an effective and safe treatment strategy for the patients with advanced PD.

Anticancer Effect and Apoptosis Mechanism of Photochemotherapy Using Photogem and 632 nm Diode Laser on Human Squamous Cell Carcinoma Cell Line / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: This study was conducted to investigate the anticancer effect of photochemotherapy in vitro using a photosensitizing agent (Photogem) and a laser therapy (632 nm diode laser). MATERIALS AND METHOD: Human squamous cell carcinoma cell (SNU-1041) was treated to laser therapy for various irradiation times, laser powers, and interval times. The treated cell was analyzed by MTT assay, DAPI staining to see apoptosis. RESULTS: The viability of cells was decreased with the increasing of the laser irradiation time and the laser power. No significant difference in cell viability was noted by various interval time. Increasing apoptosis was observed by increasing concentration of Photogem and increasing lasering time by DAPI staining. CONCLUSION: This study demonstrated anticancer effect of photochemotherapy using Photogem and 632 nm diode laser. Apoptosis was observed in the process of cancer cell death.

Treatment of Protein-losing Enteropathy After Fontan Procedure by Conversion to the Total Cavopulmonary Connection with Fenestration / 대한흉부외과학회지

As the operative mortality has diminished and the number of survivors has increased after Fontan procedure, morbidities related to the unnatural physiology of cavopulmonary flow have developed. One of the complications by the hemodynamic derangement after Fontan procedure is a protein-losing enteropathy. This is a rare but life-threatening complication after the Fontan operation. Treatment strategies are highly variable. But, reports on successful management are limited. We experienced three cases of protein-losing enteropathy after the Fontan operation. We report that the conversion to the total extracardiac or intracardiac cavopulmonary connection with fenestration is a satisfactory treatment modality for protein- losing enteropathy after the Fontan operation.

A Case of Seizure Induced by Levothyroxine Intoxication in Euthyroidism

No abstract available.

Pleural Effusion and Pancreatico-Pleural Fistula Associated withAsymptomatic Pancreatic Disease / 결핵

Effusions arising from acute pancreatitis are usually small, left sided and self limiting. The incidence of pleural effusions in acute pancreatitis is reported between 3% and 17%. In chronic pancreatitis, as a consequence of fistula and pancreatitic pseudocyst formation or by spontaneous rupture of a pancreatic psudocyst directly into thoracic cavity, extremely large effusions may be seen. When the underlying pacreatic disease is asymptomatic, the diagnosis is made by measuring the amylase content of the pleural fluid. We experience a case of left sided pleural effusions caused by pancreatico-pleural fistula associated with pancreatic pseudocyst. The diagnosis was made by measuring of pleural fluid amylase level (80000U/L). Abdominal CT scan revealed pancreatic pseudocyct and pancreatitis with extension to left pleural space through esophageal hiatus and extension to left subdiaphragmatic space. Left pleural effusions were decreased after fasting, total parenteral nutrition and percutaneous pleural fluid catheter drainage. We reported a case of pleural effusions and pacreatico-pleural fistula asssociated with asymptomatic pancreatic disease with review of literatures.

A Study of Endothelium-dependent Pulmonary Arterial Relaxation and the Role of Nitric oxide on Acute Hypoxic Pulmonary Vasoconstriction in Rats / 결핵

BACKGROUND: Since the demonstration of the fact that vascular relaxation by acetylcholine(Ach) results from the release of relaxing factor from the endothelium, the identity and physiology of this endothelium-derived relaxing factor(EDRF) has been the target for many researches. EDRF has been identified as nitric oxide(NO). With the recent evidences that EDRF is an important mediator of vascular tone, there have been increasing interests in defining the role of the EDRF as a potential mediator of hypoxic pulmonary vasoconstriction. But the role of EDRF in modulating the pulmonary circulation is not compeletely clarified. To investigate the endotbelium-dependent pulmonary vasodilation and the role of EDRF during hypoxic pulmonary vasoconstriction, we studied the effects of N(G)-monornethyl-L-arginine(L-NMMA) and L-arginine on the precontracted pulmonary arterial rings of the rat in normoxia and hypoxia. METHODS: The pulmonary arteries of male Sprague Dawley(300~350g) were dissected free of surrounding tissue, and cut into rings. Rings were mounted over fine rigid wires, in organ chambers filled with 20ml of Krebs solution bubbled with 95 percent oxygen and 5 percent carbon dioxide and maintained at 37℃. Changes in isometric tension were recorded with a force transducer(FT. 03 Grass, Quincy, USA). RESULTS: 1) Precontraction of rat pulmonry artery with intact endothelium by phenylephrine(PE, 10(-6)M) was relaxed completely by acetylcholine(Ach, 10(-9) -10(-5)M) and sodium nitroprusside (SN, 10(-9) -10(-5)M), but relaxing response by Ach in rat pulmonary artery with denuded endothelium was significantly decreased. 2) L-NMMA(10-4M) pretreatment inhibited Ach(10(-9) -10(-5)M)-induced relaxation, but L-NMMA(10-4M) had no effect on relaxation induced by SN(10(-9) -10(-5)M). 3) Pretreatment of the L-arginine(10(-4)M) significantly reversed the inhibition of the Ach(10(-9) -10(-5)M)-induced relaxation caused by L-NMMA(10(-4)M). 4) Pulmonary arterial contraction by PE(10(-6)M) was stronger in hypoxia than normoxia but relaxing response by Ach(10(-9) -10(-5)M) was decreased. 5) With pretreatment of L-arginine(10(-4)M), pulmonary arterial relaxation by Ach(10(-9) -10(-5)M) in hypoxia was reversed to the level of relaxation in normoxia. CONCLUSION: It is concluded that rat pulmonary arterial relaxation by Ach is dependent on the intact endothelium and is largely mediated by NO. Acute hypoxic pulmonary vasoconstriction is related to the suppression on NO formation in the vascular endothelium.